Connective Tissue Disease-Associated ILD

Connective tissue disease associated with interstitial lung disease, or CT-ILD, is a lung condition that affects a small number of patients with a connective tissue disease. Examples of connective tissue diseases — also known as rheumatologic, collagen vascular or autoimmune diseases — include scleroderma, rheumatoid arthritis, Sjogren's syndrome, systemic lupus erythematosus, polymyositis, dermatomyositis and mixed connective tissue disease.

Patients are often diagnosed with the connective tissue disease first and develop CT-ILD later, although in some cases, the opposite occurs.

CT-ILD causes inflammation or scarring (fibrosis) of the lungs, or both. The exact cause of lung damage is unknown.

Some patients with CT-ILD don't have symptoms. For others, common symptoms include:

  • Shortness of breath with activity
  • Cough
  • Fatigue
  • "Crackle" sound heard when listening to the chest with a stethoscope
  • Symptoms of a connective tissue disease, such as joint pain and swelling, rash, dry eyes, dry mouth and acid reflux

The diagnosis of CT-ILD requires input from pulmonologists, radiologists, and in some cases, pathologists experienced in interstitial lung disease.

In making a diagnosis, your doctor will start by conducting a thorough medical history and physical examination. This will include discussing your health history, reviewing medications and potential occupational and environmental exposures, listening to your chest with a stethoscope to check for a crackling sound, and carefully examining your skin and joints.

In addition, your doctor may conduct the following tests:

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CT-ILD is treated with anti-inflammatory or immunosuppressive medications. You may recognize some or all of these medications if they were prescribed to you for your connective tissue disease.

The most common medications used to treat CT-ILD are:

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Reviewed by health care specialists at UCSF Medical Center.